Persistent Isolated Mydriasis As an Early Sign of Internal Carotid Artery

Home , Anisocoria, Miosis, Mydriasis, Ophthalmoparesis

Clinical Neurology and Neurosurgery 182 (2019) 70–72

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Clinical Neurology and Neurosurgery

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Case Report Persistent isolated mydriasis as an early sign of internal carotid artery dissection: Pourfour du petit syndrome T ⁎ José Ramón Sánchez-de la Torrea, , Marta Drake-Pérezb, Alfonso Casadoc, Enrique Jesús Palacio-Portillaa, Marian Revillaa, José Luis Vázquez-Higueraa,d, Jon Infantea,d a Service of Neurology, University Hospital “Marqués de Valdecilla” - IDIVAL - University of Cantabria. Santander, Spain b Service of Radiology, University Hospital “Marqués de Valdecilla” - IDIVAL, Santander,Spain c Service of Ophthalmology, University Hospital “Marqués de Valdecilla” - IDIVAL, Santander, Spain d “Centro de Investigación de red de enfermedades neurodegenerativas (CIBERNED)”. Spain

ARTICLE INFO ABSTRACT

Keywords: Background: The dissection of the internal carotid artery (ICA) is commonly associated with miosis in Bernard- Cerebrovascular diseases Horner syndrome (BHS). The presence of mydriasis is exceptional but can occur in the context of Pourfour du Neuroophthalmology Petit syndrome (PDPS), a rare entity opposite of BHS accompanied by eyelid retraction and hyperhidrosis and Pourfour du Petit syndrome caused by hyperactivity of the sympathetic cervical chain. Internal carotid artery dissection Aim: To report on a case of PDPS as the first manifestation of an ICA dissection. Mydriasis Method: A 54-year-old man presented with isolated left mydriasis with no other abnormalities in the examination. Six months later, he suffered an ischemic stroke in the left middle cerebral artery territory secondary to a left ICA dissection. Results: The initial study with Intracranial computed tomographic angiography and brain magnetic resonance imaging ruled out compressive cause of the third cranial nerve or structural lesion in the midbrain. The absence of hypersensitivity to Pilocarpine discarded postganglionic parasympathetic involvement. Conclusions: In the presence of unilateral mydriasis and once common causes are ruled out an imaging examination of the supra-aortic trunks should be completed, since it could represent the first sign of carotid pathology in the context of PDPS.

1. Introduction examination (Fig. 1), the only sign was anisocoria, with left pupil larger than right and unresponsive to light (Fig. 1A and B). The visual acuity The dissection of the internal carotid artery (ICA) accounts for up to was 20/20 and 20/25 on the right and the left eye respectively. Pupil 20% of strokes in young patients and is frequently associated to head or diameters were 2 and 4 mm with photopic conditions in the right and neck pain, cerebral ischemia or Bernard-Horner syndrome (BHS) pre- left eye respectively; and 4 mm in both eyes with scotopic conditions. senting with miosis, ptosis and enophthalmos. Pourfour du Petit syn- Eye movements were normal, and there was no evidence of ptosis, drome (PDPS) is a rare entity that presents as a reverse BHS, with eyelid retraction or facial hyperhidrosis. Pharmacological ocular tests mydriasis, eyelid retraction and hyperhidrosis. Like BHS, it is caused by showed a negative response to Pilocarpine 0.125% but a signiﬁcant injuries in the sympathetic cervical chain [1]. We report on the case of a contraction of the mydriatic pupil to the administration of Pilocarpine patient with PDPS that presented with unilateral persistent isolated at 1% (Fig. 1C), indicating either sympathetic overactivity or pregan- mydriasis six months prior to an ischemic stroke secondary to a spon- glionar parasympathetic underactivity. Due to the main suspicion of taneous ICA dissection. Previous literature on the topic is reviewed. non-reactive mydriasis as an initial symptom of a compressive III cranial nerve palsy, an intracranial computed tomographic angiography 2. Case report (CTA) at the level of the Circle of Willis was performed in order to rule out a posterior communicating artery aneurysm. No imaging of the A 54-year-old man had sudden painless blurred vision. He reported supra-aortic trunks, including cervical carotid artery, was obtained at no current or previous history of headache, neck pain or trauma. On the that moment. Besides, a brain magnetic resonance imaging (MRI) was

⁎ Corresponding author. E-mail address: [email protected] (J.R. Sánchez-de la Torre). https://doi.org/10.1016/j.clineuro.2019.04.030 Received 6 January 2019; Received in revised form 19 April 2019; Accepted 30 April 2019 Available online 01 May 2019 0303-8467/ © 2019 Elsevier B.V. All rights reserved. J.R. Sánchez-de la Torre, et al. Clinical Neurology and Neurosurgery 182 (2019) 70–72

tomography showed a hyperacute ischemic stroke involving the left middle cerebral artery (MCA) territory. The CTA showed a dissection of the left internal carotid artery (ICA) on its extracranial portion (Fig. 2) and a distal thrombus placed at the proximal segment of the ipsilateral MCA. Treatment consisted on intravenous ﬁbrinolysis, mechanical thrombectomy and carotid stenting. The clinical evolution was good. Left eye mydriasis persisted on the last examination 9 months from onset.

3. Discussion

PDPS is a somewhat unknown entity also known as reverse Horner syndrome. This term was coined for the first time by Biffi in 1846 in honor of Pourfour du Petit, a French military surgeon [1]. The syndrome is characterized by unilateral mydriasis, eyelid retraction, and abnormal sweating response with hyperhidrosis. However, the clinical manifestations vary from cases with complete PDPS to others with isolated mydriasis or hyperhidrosis. The clinical course is variable, from transitory self-limited cases to permanent ones. The exact mechanism is not clear, but it is believed to be due to a sympathetic hyperstimulation caused by an irritation of the cervical sympathetic chain [1]. Various pathologies affecting the cervical sympathetic pathway have been associated with this syndrome: Nonpenetrating injuries of the cervical spine, traumatic pseudo-aneurysm of the internal carotid artery, cervical and thoracic tumors, and iatrogenic causes, such as complications after central venous catheterization or positional post- surgical side-effect. Fig. 1. Pupillary findings: Anisocoria due to left mydriasis in ambient light There is a close relationship between PDPS and BHS, indeed the (1A). The dilated pupil is not reactive to light (1B). Post 1% pilocarpine shows a same etiologies can produce any of both conditions. It is believed that left pupil contraction (1C). PDPS would be the clinical pattern whenever the insult irritates the sympathetic fibers, while BHS would be associated to deficitary states. It is however not possible to predict which phenomenon will occur after done in order to assess the upper brainstem and basal cisterns. Both a certain damage. examinations showed no significant findings. Antiplatelet therapy was Although the causes of a unilateral mydriasis are multiple, including added. On a follow-up visit four months later, the left mydriasis per- compressive or ischemic neuropathy of the III cranial nerve and mi- sisted without any change. graine, only few cases of isolated mydriasis as the first symptom of a After 6 months, the patient suffered a sudden onset right hemi- carotid dissection have been reported [2]. The dissection of ICA is often plegia, aphasia and right homonymous hemianopsia. Cranial computed associated to ocular pathology like amaurosis fugax and BHS. Mydriasis

Fig. 2. Computed tomographic angiography performed in the Emergency Department shows a progressive narrowing of the eccentric lumen of the left internal carotid artery, surrounded by a crescent-shaped mural thrombus, with complete distal obstruction. This is known as the “string sign” and suggests a dissection (arrow in 2A). Diﬀusion weighted imaging of the magnetic resonance imaging performed in the acute phase shows areas of restricted diﬀusion in the posterior territory of the left middle cerebral artery representing an acute stroke (2B).

71 J.R. Sánchez-de la Torre, et al. Clinical Neurology and Neurosurgery 182 (2019) 70–72 is exceptional and attributed to either a sympathetic overactivation or a therefore crucial to recognize its atypical neuro-ophthalmological parsympathetic underactivation. Rarely, a carotid pathology can pro- manifestations such as the Pourfour du Petit Syndrome. Although the duce a parasympathetic underactivation triggering a unilateral my- symptoms are opposed to those of Horner’s syndrome, it has the same driasis. In some of these cases the mydriasis is usually followed by diagnostic value. Importantly, as shown here, it might precede in ophthalmoparesis, due to the damage of the third cranial nerve by months the appearance of other symptoms due to ICA dissection. Then, hemodynamic mechanisms [3]. Also, a compressive mechanism due to in the presence of unilateral mydriasis, once ruled out commonest compensatory hypertrophy of the posterior ipsilateral communicating causes, CTA of the supra-aortic trunks should be performed. artery in an ICA agenesia case has been reported [4]. Both causes were ruled out in our case given the normality of CTA and the absence of Conflicts of interest concomitant ophthalmoparesis. Other times, transient mydriasis is associated with a parasympathetic dysfunction secondary to ischemia in The authors declare that they have no conflict of interest. the ciliary ganglion, a structure irrigated by the ophthalmic artery through the ciliary arteries. ICA dissection is reported cause of ischemia Ethical standard of this structure [5]. This etiology was also discarded in our patient given the absence of hypersensitivity to low doses of pilocarpine This study was performed in accordance with ethical standards, and (0.125%) on eyedrops testing which is characteristically observed in informed consent was waived. cases of a tonic pupil. In our case, we propose that the spontaneous dissection of the in- References ternal carotid artery affected in the first term the cervical sympathetic pathway, causing the irritation of the oculo-sympathetic fibers. This [1] A.E. Best, Pourfour du Petit’s experiments on the origin of the sympathetic nerve, was confirmed by the response to Pilocarpine at 1% but not at 0,125%. Med. Hist. 13 (2) (1969) 154–174. fi [2] R. Inzelberg, P. Nisipeanu, S.C. Blumen, I. Kovach, G.M. Groisman, R.L. Carasso, The long interval between the onset of the rst symptoms of ICA dis- Transient unilateral mydriasis as the presenting sign of aortic and carotid dissection, section and the secondary stroke in this case is not such unusual since Neurology 55 (12) (2000) 1934–1935, https://doi.org/10.1212/WNL.55.12.1934-a. widely variable intervals ranging from hours to months have been re- [3] H. Koennecke, S. Seyfert, Mydriatic pupil as the presenting sign of common carotid artery dissection, Stroke 29 (12) (1998) 2653–2655, https://doi.org/10.1161/01. ported in the literature. In our case, the stable degree of mydriasis, even STR.29.12.2653. after correct treatment with carotid stenting, could be explained by an [4] M. Ibrahim, H.M. Branson, J.R. Buncic, M.M. Shroff, A case of Horner syndrome with aberrant regeneration of the oculosympathetic pathway, as suggested in intermittent mydriasis in a patient with hypoplasia of the internal carotid artery, – previous literature. AJNR Am. J. Neuroradiol. 27 (6) (2006) 1318 1320. [5] G.K. Sharma, V.R. Deshmukh, F.C. Albuquerque, T.R. Wolf, C.G. McDougall, Resolution of mydriatic pupil after angioplasty and stenting of cervical internal 4. Conclusions carotid artery dissection: case report, Neurosurgery 64 (3) (2009) E562–563, https:// doi.org/10.1227/01.NEU.0000338596.33446.06 discussion E3. Internal carotid artery dissection is a serious condition and it is